● Swelling, tenderness and stiffness of an extremity, often lower forearm
● May be associated with carpal tunnel syndrome
● Inflammatory arthritis, contractures and subclinical myositis have been documented
● Esophageal dysmotility, pericardial and pleural effusion, lung and kidney involvement
● The association with serious hematological abnormalities has led to the suggestion that all patients with this disease
● Should have a bone marrow examination to exclude myeodysplasia

● Mild chronic inflammatory cell infiltrate consisting of lymphocytes, plasam cells, histiocytes, and variable numbers of eosinophils may be present in the deep reticular dermis, which is also fibrosed with atrophy of sweat glands
● Occasionally the dermal changes may not be distinguished from Morphea
● The most dramatic changes are found in the superficial fascia which is markdly thickened, fibrosed and sclerotic
● A chronic inflammatory cell infiltrate is present both within the fascia and around blood vessels
● Tissue eosinophilia is focal and often transitory
● Its absence in no way preclude the diagnosis
● Lymphoid follicles with germinal centre are also occasionally evident

● Immunofluorescence has revealed deposition of IgM at the dermo –epidermal junction, immunoglobulin and complement around blood vessels in the deep dermis and IgG and complement in the deep fascia and skeletal muscle

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